Management of cerebrospinal fluid pseudocysts in the laparoscopic age.

OBJECTIVE: Abdominal CSF pseudocysts are an uncommon but challenging complication of ventriculoperitoneal shunts. Pseudocysts consist of a loculated intraperitoneal compartment that inadequately absorbs CSF and may be infected or sterile at diagnosis. The treatment goal is to clear infection if present, reduce inflammation, and reestablish long-term function in an absorptive (intraperitoneal) space. This aim of this paper was to study the efficacy of primary laparoscopic repositioning of the distal shunt catheter for treatment of sterile abdominal CSF pseudocysts. METHODS: All patients treated for abdominal CSF pseudocysts at Dallas Children's Health from 1991 to 2021 were retrospectively reviewed. Patient history and pseudocyst characteristics were analyzed, with a primary outcome of pseudocyst recurrence at 1 year. RESULTS: Of 92 primary pseudocysts, 5 initial treatment strategies (groups) were used depending on culture status, clinical history, and surgeon preference: 1) shunt explant/external ventricular drain (EVD) placement (23/92), 2) distal tubing externalization (13/92), 3) laparoscopic repositioning (35/92), 4) open repositioning (4/92), and 5) other methods such as pseudocyst drainage or direct revision to another terminus (17/92). Seventy pseudocysts underwent shunt reimplantation in the peritoneal space. The 1-year peritoneal shunt survival for groups 1 and 2 combined was 90%, and 62% for group 3. In group 3, 1-year survival was better for those with normal systemic inflammatory markers (100%) than for those with high markers (47%) (p = 0.042). In a univariate Cox proportional hazards model, the risk of pseudocyst recurrence was increased if the most recent abdominal procedure was a nonshunt abdominal surgery (p = 0.012), and it approached statistical significance with male sex (p = 0.054) and elevated inflammatory markers (p = 0.056. Multivariate Cox analysis suggested increased recurrence risk with male sex (p = 0.05) and elevated inflammatory markers (p = 0.06), although the statistical significance threshold was not reached. The length of hospital stay was shorter for laparoscopic repositioning (6 days) than for explantation/EVD placement (21 days) (p < 0.0001). Ultimately, 62% of patients had a peritoneal terminus at the last follow-up, 33% (n = 30) had an extraperitoneal terminus (19 pleura, 8 right heart, and 3 gallbladder), and 5 patients were shunt free. CONCLUSIONS: Some sterile pseudocysts with normal systemic inflammatory markers can be effectively treated with laparoscopic repositioning, resulting in a significantly shorter hospitalization and modestly higher recurrence rate than shunt explantation.

Hypoplasia of Extrahepatic Biliary Tree and Intrahepatic Cholangiolopathy in Cystic Fibrosis Imperfectly Mimic Biliary Atresia in 4 Infants With Cystic Fibrosis and Kasai Portoenterostomy.

Four male infants with cystic fibrosis and prolonged neonatal jaundice underwent Kasai procedure to relieve biliary obstruction due to apparent biliary atresia. The excised remnants had viscid mucus accumulation in hypoplastic gallbladders and distended peribiliary glands. Main hepatic ducts were narrow and/or malformed. Microscopic differences between the gallbladder and extrahepatic bile ducts in cystic fibrosis and sporadic biliary atresia were unequivocal, despite some histologic overlap; no erosive or fibro-obliterative lesions typical of biliary atresia were seen. Common in liver, biopsies were small duct cholangiopathy with intense focal cholangiolitis and massive accumulation of ceroid pigment within damaged cholangiocytes, and in portal macrophages, portal fibrosis, and unequivocal features of large duct obstruction were inconspicuous compared with biliary atresia. Plugs of bile in small ducts tended to be pale and strongly periodic acid-Schiff-reactive in cystic fibrosis. Distinguishing the liver lesion from that of biliary atresia is challenging but possible. Liver biopsies from 2 additional infants with cystic fibrosis and prolonged jaundice that spontaneously resolved showed a similar small duct cholangiopathy. Small gallbladders and extrahepatic ducts challenge surgical judgment as findings in liver biopsies challenge the pathologist. The decision to perform a Kasai procedure is reasonable when mimicry of biliary atresia is grossly complete. We hypothesize that a disorder of bile volume/flow during development and/or early infancy linked to the CFTR mutation alone or in combination with the stresses of neonatal intensive care causes destructive cholangiolitis and intrahepatic reduction of bile flow with secondary hypoplasia of extrahepatic biliary structures.

Esophagus-Like Bronchus: A Noncommunicating Bronchopulmonary Foregut Malformation.

We describe a previously unreported bronchopulmonary foregut malformation wherein a segment of a bronchus of the lower lobe of the left lung in a 4-year-old girl was entirely esophageal in structure. No communication was identified between the tracheobronchial tree and the esophagus by radiologic examination or at surgery. The esophagus-like bronchus was associated with an adjacent atretic bronchus and a downstream cavity in the lower lobe of the left lung. The child sought clinical attention because of recurrent pulmonary infections localized to the lower lobe of the lung. We posit that this esophagus-like bronchus is a novel noncommunicating bronchopulmonary foregut malformation.

ACTG2-Associated Visceral Myopathy With Chronic Intestinal Pseudoobstruction, Intestinal Malrotation, Hypertrophic Pyloric Stenosis, Choledochal Cyst, and a Novel Missense Mutation.

Primary visceral myopathy caused by a pathogenic mutation in the gene encoding the enteric smooth muscle actin gamma 2 ( ACTG2) affects gastrointestinal and genitourinary tracts and often presents as chronic intestinal pseudoobstruction. We present a case of pediatric onset chronic intestinal pseudoobstruction associated with a novel missense ACTG2 mutation c.439G>T/p.G147C. In addition to the known disease manifestations of feeding intolerance and intestinal malrotation, our patient had a late-onset hypertrophic pyloric stenosis and a late-onset choledochal cyst, the former of which has not previously been described in patients with ACTG2-associated visceral myopathy.

Craniectomy and Traumatic Brain Injury in Children on Extracorporeal Membrane Oxygenation Support.

Severe trauma may cause refractory life-threatening respiratory failure requiring extracorporeal membrane oxygenation (ECMO). Concurrent traumatic brain injury, however, complicates the use of ECMO because of the major risk of intracranial bleeding with systemic anticoagulation. Craniotomy and/or craniectomy for hematoma evacuation during ECMO are extremely high-risk procedures secondary to ongoing anticoagulation, and there are only a few such case reports in the literature. We present the case of a child with multiple thoracic injuries and life-threatening respiratory failure supported on ECMO. She developed an intracranial hemorrhage while systemically heparinized that required emergent decannulation and bedside craniectomy for hematoma extraction. She survived with an excellent neurologic outcome. We also review the relevant literature regarding the use of ECMO in patients with polytrauma and the occurrence of craniectomy on extracorporeal support, with a focus on pediatric publications. Patients with polytrauma with brain injury can be supported on ECMO, but extreme precaution must be taken regarding anticoagulation. The intracranial complications of ECMO in this population are not infrequent, but our case report and review of the literature suggest that neurosurgical intervention should be considered in life-threatening conditions when no other alternatives are available.

Does restraint status in motor vehicle crash with rollover predict the need for trauma team presence on arrival? An ATOMAC study.

PURPOSE: Restraint status has not been combined with mechanistic criteria for trauma team activation. This study aims to assess the relationship between motor vehicle crash rollover (MVC-R) mechanism with and without proper restraint and need for trauma team activation. METHODS: Patients <16years old involved in an MVC-R between November 2007 and November 2012 at 6 Level 1 pediatric trauma centers were included. Restraint status, the need for transfusion or intervention in the emergency department (ED), hospital and intensive care length of stay and mortality were assessed. RESULTS: Of 690 cases reviewed, 48% were improperly restrained. Improperly restrained children were more likely to require intubation (OR 10.24; 95% CI 2.42 to 91.69), receive blood in the ED (OR 4.06; 95% CI 1.43 to 14.17) and require intensive care (ICU) (OR; 3.11; 95% CI 1.96 to 4.93) than the properly restrained group. The improperly restrained group had a longer hospital length of stay (p<0.001), and a higher mortality (3.4% vs. 0.8%; OR 4.09; 95% CI 1.07 to 23.02) than the properly restrained group. CONCLUSION: Unrestrained children in MVC-R had higher injury severity and were significantly more likely to need urgent interventions compared to properly restrained children. This supports a modification to include restraint status with the rollover criterion for trauma team activation.

Multicenter study of pectus excavatum, final report: complications, static/exercise pulmonary function, and anatomic outcomes.

BACKGROUND: A multicenter study of pectus excavatum was described previously. This report presents our final results. STUDY DESIGN: Patients treated surgically at 11 centers were followed prospectively. Each underwent a preoperative evaluation with CT scan, pulmonary function tests, and body image survey. Data were collected about associated conditions, complications, and perioperative pain. One year after treatment, patients underwent repeat chest CT scan, pulmonary function tests, and body image survey. A subset of 50 underwent exercise pulmonary function testing. RESULTS: Of 327 patients, 284 underwent Nuss procedure and 43 underwent open procedure without mortality. Of 182 patients with complete follow-up (56%), 18% had late complications, similarly distributed, including substernal bar displacement in 7% and wound infection in 2%. Mean initial CT scan index of 4.4 improved to 3.0 post operation (severe >3.2, normal = 2.5). Computed tomography index improved at the deepest point (xiphoid) and also upper and middle sternum. Pulmonary function tests improved (forced vital capacity from 88% to 93%, forced expiratory volume in 1 second from 87% to 90%, and total lung capacity from 94% to 100% of predicted (p < 0.001 for each). VO2 max during peak exercise increased by 10.1% (p = 0.015) and O2 pulse by 19% (p = 0.007) in 20 subjects who completed both pre- and postoperative exercise tests. CONCLUSIONS: There is significant improvement in lung function at rest and in VO2 max and O2 pulse after surgical correction of pectus excavatum, with CT index >3.2. Operative correction significantly reduces CT index and markedly improves the shape of the entire chest, and can be performed safely in a variety of centers.

Duodenal injuries in the very young: child abuse?

BACKGROUND: Duodenal injuries in children are uncommon but have been specifically linked with child abuse in case reports. Owing to the rarity of the diagnosis, few studies to date have looked at the association between duodenal injuries and mechanism in younger child. We hypothesize that duodenal injuries in the very young are significantly associated with child abuse. METHODS: This investigation is a retrospective cohort study of patients admitted with duodenal injuries at one of six Level I pediatric trauma centers. All institutions had institutional review board approval. The trauma registries were used to identify children aged 0 year to 5 years from 1991 to 2011. Multiple variables were collected and included age, mechanism of injury, type of duodenal injury, additional injuries, mortality, and results of abuse investigation if available. Relationships were analyzed using Fischer's exact test. RESULTS: We identified 32 patients with duodenal injuries with a mean age of 3 years. Duodenal injuries included duodenal hematomas (44%) and perforations/transections (56%). Of all duodenal injuries, 53% resulted in operation, 53% had additional injuries, and 12.5% resulted in death. Of the 32 children presenting with duodenal injuries, 20 were child abuse patients (62.5%). All duodenal injuries in children younger than 2 years were caused by child abuse (6 of 6, p = 0.06) and more than half of the duodenal injuries in children older than 2 years were caused by child abuse (14 of 26). Child abuse-related duodenal injuries were associated with delayed presentation (p = 0.004). There was a significant increase in child abuse-related duodenal injuries during the time frame of the study (p = 0.002). CONCLUSION: Duodenal injuries are extremely rare in the pediatric population. This multi-institutional investigation found that child abuse consistently associated with duodenal injuries in children younger than 2 years. The evidence supports a child abuse investigation on children younger than 2 years with duodenal injury. LEVEL OF EVIDENCE: Epidemiological study, level III.

Hand ischemia associated with elbow trauma in children.

OBJECTIVES: Previous observational studies suggest that children with hand ischemia following elbow trauma can be safely observed if Doppler signals are present in the wrist arteries (pink pulseless hand, PPH). Nonoperative management of PPH is predicated on the assumption that PPH results from local arterial spasm, but the mechanism of arterial compromise has not been investigated. We hypothesized that PPH signifies a brachial artery injury that requires surgical repair. METHODS: Retrospective review of operations performed on children with hand ischemia following elbow trauma at a level I trauma center pediatric hospital. RESULTS: Between 2003 and 2010, 12 children (seven males, mean age 7.4 years) underwent brachial artery exploration for hand ischemia following elbow trauma (11 supracondylar fractures, one elbow dislocation) due to falls (n = 10) or motor vehicle crashes (n = 2). At presentation, three subjects had normal radial pulses, eight subjects had Doppler signals but no palpable pulses, and one had weak Doppler flow with advanced hand ischemia. Six of the nine subjects without palpable pulses also had neurosensory changes. All 12 subjects underwent brachial artery exploration either initially (n = 2) or following orthopedic fixation (n = 10) due to persistent pulselessness. At operation, eight of 12 patients (67%) had focal brachial artery thrombosis due to intimal flaps, and four had brachial artery and median nerve entrapment within the pinned fracture site. At discharge, all 12 subjects had palpable radial pulses, but three with entrapment had dense median nerve deficits. One of the three subjects with dense neurologic deficit had complete recovery of neurologic function at ten months. The other two subjects had residual median nerve deficits with partial recovery at 5 and 6 months follow-up, respectively. No patient developed Volkman's contracture. CONCLUSIONS: Brachial artery injuries should be anticipated in children with hand ischemia associated with elbow trauma. Neurovascular entrapment at the fracture site is a possible complication of orthopedic fixation. Absence of palpable wrist pulses after orthopedic fixation should prompt immediate brachial artery exploration. PPH should not be considered a consequence of arterial spasm in these patients.

Increasing severity of pectus excavatum is associated with reduced pulmonary function.

OBJECTIVE: To determine whether pulmonary function decreases as a function of severity of pectus excavatum, and whether reduced function is restrictive or obstructive in nature in a large multicenter study. STUDY DESIGN: We evaluated preoperative spirometry data in 310 patients and lung volumes in 218 patients aged 6 to 21 years at 11 North American centers. We modeled the impact of the severity of deformity (based on the Haller index) on pulmonary function. RESULTS: The percentages of patients with abnormal forced vital capacity (FVC), forced expiratory volume in 1 second (FEV(1)), forced expiratory flow from 25% exhalation to 75% exhalation, and total lung capacity findings increased with increasing Haller index score. Less than 2% of patients demonstrated an obstructive pattern (FEV(1)/FVC <67%), and 14.5% demonstrated a restrictive pattern (FVC and FEV(1) <80% predicted; FEV(1)/FVC >80%). Patients with a Haller index of 7 are >4 times more likely to have an FVC of ≤80% than those with a Haller index of 4, and are also 4 times more likely to exhibit a restrictive pulmonary pattern. CONCLUSIONS: Among patients presenting for surgical repair of pectus excavatum, those with more severe deformities have a much higher likelihood of decreased pulmonary function with a restrictive pulmonary pattern.

Fetal magnetic resonance imaging in isolated diaphragmatic hernia: volume of herniated liver and neonatal outcome.

OBJECTIVE: We sought to use magnetic resonance (MR) imaging (MRI) to estimate percentage of fetal thorax occupied by lung, liver, and other abdominal organs in pregnancies with congenital diaphragmatic hernia (CDH). STUDY DESIGN: This was a retrospective study of pregnancies with isolated CDH referred for MRI between August 2000 and June 2006. Four regions of interest were measured in the axial plane by an investigator blinded to neonatal outcome, and volumes were then calculated. The percentages of thorax occupied by lung, liver, and all herniated organs were then compared with neonatal outcomes. RESULTS: Fifteen CDH fetuses underwent MRI at a median gestational age of 29 weeks. Liver herniation was found in 93%. When the liver occupied > 20% of the fetal thorax, neonatal deaths were significantly increased. Percentages of lung and other herniated organs were not associated with outcome. CONCLUSION: In our MR series of isolated CDH, neonatal deaths were significantly increased when > 20% of the fetal thorax was occupied by liver.

Surgical repair of pectus excavatum markedly improves body image and perceived ability for physical activity: multicenter study.

OBJECTIVE: This study evaluated changes in both physical and psychosocial quality of life reported by the parent and child after surgical repair of pectus excavatum. METHODS: As part of a multicenter study of pectus excavatum, a previously validated tool called the Pectus Excavatum Evaluation Questionnaire was administered by the research coordinator, via telephone, to parents and patients (8-21 years of age) before and 1 year after surgery. Eleven North American children's hospitals participated. From 2001 to 2006, 264 patients and 291 parents completed the initial questionnaire, and 247 patients and 274 parents completed the postoperative questionnaire. Responses used a Likert-type scale of 1 to 4, reflecting the extent or frequency of a particular experience, with higher values conveying less-desirable experience. RESULTS: Preoperative psychosocial functioning was unrelated to objective pectus excavatum severity (computed tomographic index). Patients and their parents reported significant positive postoperative changes. Improvements occurred in both physical and psychosocial functioning, including less social self-consciousness and a more-favorable body image. For children, the body image component improved from 2.30+/-0.62 (mean+/-SD) to 1.40+/-0.42 after surgery and the physical difficulties component improved from 2.11+/-0.82 to 1.37+/-0.44. For the parent questionnaire, the child's emotional difficulties improved from 1.81+/-0.70 to 1.24+/-0.36, social self-consciousness improved from 2.86+/-1.03 to 1.33+/-0.68, and physical difficulties improved from 2.14+/-0.75 to 1.32+/-0.39. Ninety-seven percent of patients thought that surgery improved how their chest looked. CONCLUSIONS: Surgical repair of pectus excavatum can significantly improve the body image difficulties and limitations on physical activity experienced by patients. These results should prompt physicians to consider the physiologic and psychological implications of pectus excavatum just as they would any other physical deformity known to have such consequences.

Outcomes of pregnancies with fetal gastroschisis.

OBJECTIVE: To describe pregnancy outcomes with fetal gastroschisis, including the associations of prenatal ultrasound findings with neonatal surgical complications and other morbidities. METHODS: This was a review of pregnancies complicated by fetal gastroschisis and delivered from January 1998 through June 2006. The last ultrasonogram before delivery was reviewed to determine stomach dilatation, bowel dilatation, or abnormalities of amniotic fluid volume. Neonatal records were reviewed to determine type of closure and any bowel complications. RESULTS: There were 66 pregnancies with gastroschisis, 1 per 2,000 deliveries. There were three stillbirths and three neonatal deaths. Delayed closure was necessary in 49% who underwent surgery. Birth weight below the third percentile, which occurred in 38%, was associated with need for delayed closure, 64% compared with 25% without growth restriction, P<.001, but was not associated with longer hospital stay or neonatal death. Fetal gastroschisis was diagnosed by prenatal ultrasonography in 58 cases. Bowel complications requiring surgery were more frequent when ultrasonography had demonstrated stomach dilatation (five cases), 60% compared with 10%, P=.002. Fetuses with defects so large that no normal ventral wall could be visualized ultrasonographically (three cases) were at increased risk for neonatal death, 100% compared with 0%, P<.001. CONCLUSION: Ultrasound findings associated with adverse outcome in fetal gastroschisis included stomach dilatation and a defect so large that no normal ventral wall could be visualized. Fetal growth restriction was common, and such infants were more likely to require delayed gastroschisis closure. Despite more than 90% survival, morbidity with gastroschisis remains high. LEVEL OF EVIDENCE: II.

Prospective multicenter study of surgical correction of pectus excavatum: design, perioperative complications, pain, and baseline pulmonary function facilitated by internet-based data collection.

BACKGROUND: Given widespread adoption of the Nuss procedure, prospective multicenter study of management of pectus excavatum by both the open and Nuss procedures was thought desirable. Although surgical repair has been performed for more than 50 years, there are no prospective multicenter studies of its management. STUDY DESIGN: This observational study followed pectus excavatum patients treated surgically at 11 centers in North America, according to the method of choice of the patient and surgeon. Before operation, all underwent evaluation with CT scan, pulmonary function tests, and body image survey. Data were collected about associated conditions, hospital complications, and perioperative pain. One year after completion of treatment, patients will repeat the preoperative evaluations. This article addresses early results only. RESULTS: Of 416 patients screened, 327 were enrolled; 284 underwent the Nuss procedure and 43 had the open procedure. Median preoperative CT index was 4.4. Pulmonary function testing before operation showed mean forced vital capacity of 90% of predicted values; forced expiratory volume in 1 second (FEV(1)), 89% of predicted; and forced expiratory flow during the middle half of the forced vital capacity (FEF(25% to 75%)), 85% of predicted. Early postcorrection results showed that operations were performed without mortality and with minimal morbidity at 30 days postoperatively. Median hospital stay was 4 days. Postoperative pain was a median of 3 on a scale of 10 at time of discharge; the worst pain experienced was the same as was expected by the patients (median 8), and by 30 days after correction or operation, the median pain score was 1. Because of disproportionate enrollment and similar early complication rates, statistical comparison between operation types was limited. CONCLUSIONS: Anatomically severe pectus excavatum is associated with abnormal pulmonary function. Initial operative correction performed at a variety of centers can be completed safely. Perioperative pain is successfully managed by current techniques.